Bronquiectasias: ¿no es más una enfermedad huérfana? / Bronchiectasis: is it no longer considered an orphan disease?

Bronchiectasis, so far considered an orphan disease, currently is diagnosed with a higher frequency due to several reasons such as renewed awareness of the disease, better diagnosis including imagenology, the development of patients registries, as well as a higher number of clinical research studies. The pathological basis of bronchiectasis is widely variable. Also the clinical expression is variable, from absence of symptoms in some patients up to chronic sputum production in others. Furthermore, a group of patients often develop recurrent exacerbations. Despite the etiologies of bronchiectasis are diverse, the main etiology is previous pulmonary infection. On the other hand, bronchiectasis could also be the expression of diverse systemic diseases. Even around one quarter of patients the etiology would not be established. The development of large registries of patients has allowed the building of classifications systems with accurate prognostic criteria. Chronic infection is the most relevant issue in bronchiectasis. Infection with P. aeruginosa has been associated with poor prognosis and their eradication must be attempted always. Effective secretions drainage techniques, oral and nebulized antibiotics, as well as mucolytic therapy are the mainstay of treatment in bronchiectasis.

Las bronquiectasias, consideradas hasta un tiempo atrás una enfermedad huérfana, se diagnostican actualmente con mayor frecuencia debido a un renovado interés en esta patología, a una mejoría de técnicas de diagnóstico, existencia de mejores registros, acceso a mejores imágenes y aumento de los estudios clínicos. El sustrato anátomo-patológico es notablemente variable, al igual que la expresión clínica, que va desde la ausencia de síntomas hasta la presencia de broncorrea crónica. A su vez, un grupo de pacientes tiende a presentar exacerbaciones frecuentes. Las etiologías de las bronquiectasias son múltiples, siendo la más frecuente la existencia de infecciones pulmonares previas. También pueden formar parte de enfermedades crónicas sistémicas. Sin embargo, en 25% de los casos no es posible identificar la etiología. El desarrollo de registros de pacientes ha permitido construir modelos de clasificación de gravedad, lo que hace posible establecer criterios pronósticos. La infección crónica es un hecho frecuente en bronquiectasias y la presencia de P aeruginosa confiere mal pronóstico a la enfermedad. La erradicación de Pseudomonas debe ser intentada siempre en estos pacientes. El uso de técnicas de drenaje, los antibióticos orales y nebulizados y las terapias mucolíticas constituyen los pilares centrales en el manejo de la enfermedad.

Clinico-pathological Analysis of the Lungs from Patients with Lung Transplantation in a Single Institute in Korea

Recently, the numbers of lung transplantation (LT) has been increased in Korea. However, post-LT outcome has not been successful in all patients, which may be partially affected by the primary lung disease. Therefore comprehensive understanding in original pathological diagnosis of patients with LT would be needed for achieving better clinical outcome. To address this issue, we performed clinico-pathological analysis of the explanted lungs from 29 patients who underwent LT over a 9-yr period in Seoul National University Hospital. Among them, 26 patients received single (1/26) or double (25/26) LT, while heart-lung transplantation was performed in 3 patients. The final clinico-pathological diagnoses were idiopathic pulmonary fibrosis/usual interstitial pneumonia (UIP) (n = 6), acute interstitial pneumonia (AIP)/diffuse alveolar damage (DAD) (n = 4), AIP/non-specific interstitial pneumonia with DAD (n = 1), collagen vascular disease-related interstitial lung disease (CVD-ILD)/DAD (n = 3), CVD-ILD/UIP (n = 1), lymphangioleiomyomatosis (n = 1), bronchiectasis (n = 4), pulmonary arterial hypertension (n = 2), tuberculosis (n = 1), bronchiolitis obliterans (BO) (n = 1), and lung cancer (n = 1). Moreover, 4 patients who had chemotherapy and hematopoietic stem cell transplantation due to hematologic malignancy showed unclassifiable interstitial pneumonia with extensive fibrosis in the lungs. Our study demonstrates that pathology of the explanted lungs from Korean patients with LT is different from that of other countries except for interstitial lung disease and bronchiectasis, which may be helpful for optimization of selecting LT candidates for Korean patients.

Estudio microbiológico y anatomopatológico de bronquiectasias sangrantes en piezas de resección pulmonar / Microbiological and pathological study of leeding bronchiectasis removed by pulmonary resection

La hemoptisis periódica o recurrente en los pacientes con bronquiectasias sangrantes, secuelas de tuberculosis pulmonar, sigue siendo un factor de alto riesgo de muerte, por el desconocimiento de la comunidad médica de que existen otros agentes oportunistas que producen el sangrado, ajenos a la tuberculosis pulmonar. Determinar los verdaderos agentes causales de la infección de las bronquiectasias sangrantes. Determinar las causas del sangrado que puede ser mortal. Se han estudiado las piezas operatorias de 24 pacientes con hemoptisis por bronquiectasias sangrantes por secuelas de tuberculosis pulmonar, a quienes se le ha realizado previamente los siguientes estudios: radiografía de tórax standard y tomografía axial computarizada, examen directo y cultivo de BK, broncofibroscopia para observación directa de lesiones intraluminales sangrantes, y para determinar el nivel del sangrado con el fin de que el paciente sea sometido a cirugía para la extirpación del segmento o lóbulo sangrante. Los hallazgos del acto operatorio, han sido tomados en cuenta y las piezas operatorias han sido sometidas a un estudio anatomo-patológico y microbiológico en búsquedade hongos, tuberculosis, gérmenes comunes y neoplasia pulmonar. Se demostró la presencia del hongo Aspergillus en el 83,3% de los casos de pacientes con bronquiectasias o cavernas; el estudio de gérmenes comunes aerobios y de tuberculosis, concomitante, fue negativo en el 100% de los casos. El reporte operatorio y el estudio anatomo patológico, demuestran la presencia de micetoma y lesiones cicatriciales altamente sangrantes que hacen un acto operatorio de tiempo prolongado...

Periodical bleeding in patients with bronchiectasia, sequel of lung tuberculosis, carries a high death risk due to the fact that the medical community is not aware that other opportunistic agents, apart from tuberculosis, can produce hemorrhages. To establish the pathogenic agents of the infection in bleeding bronchiectasia from samples obtained through surgical resection. We have studied the surgical specimens obtained from 24 patients with bleeding bronchiectasia. Before operation the patients were subjected to standard radiological and computerized tomography of the lungs, directed sputum examination and culture for mycobacterium tuberculosis, as well as fiber optic bronchoscope to observe the bleeding intraluminary lesions and to establish the level of the hemorrhage, so that the patient may be operated expend to remove the bleeding segment or lobe. The surgical specimens were examined pathologically and microbiologically to detect tuberculosis, fungi, common bacteria and neoplasia. Aspergillum was found in 83.3% of the bronchiectasia or caverns. The investigation of common aerobic microbes and mycobacterium tuberculosis was negative in 100% of the specimens. Mycetoma and highly weeding ears were documented by the surgical report and pathological examination. These bleeding lesions required prolonged operative procedures. In bleeding bronchiectasia the only infective agent found was Aspergillum. There was not concomitant tuberculosis or other pathogenic agents. The invaded scar tissue was highly vascularizado causing moderate to severe bleeding, a death risk for patients.

HRCT score in bronchiectasis: Correlation with pulmonary function tests and pulmonary artery pressure

High resolution CT scan [HRCT] and its score have an important role in delineating pathological changes and pulmonary functional impairment in patients with bronchiectasis. To assess pulmonary function tests [PFTs] in patients with cystic and cylindrical bronchiectasis. To correlate HRCT score with PFTs and systolic pulmonary artery pressure [SPAP] in both radiological types. A cross-sectional study of patients with bronchiectasis diagnosed by HRCT was conducted at King Khalid University Hospital, Riyadh, Saudi Arabia. PFTs, HRCT score and SPAP were measured in both types. We studied 94 patients with bronchiectasis: 62 were cystic and 32 were cylindrical. Their mean age was 53.4 +/- 17.5 SD years. Forced vital capacity [FVC%] and forced expiratory volume in 1 second [FEV1%] were significantly lower in cystic patients [P < .0001] compared with cylindrical and Diffusion capacity [DLCO%] was also significantly lower [p < 0.01]. In cystic group PaO[2] was significantly lower and PaCO[2] higher [P < 0.0001]. HRCT score was correlated with FEV1% [r= -0.51]. HRCT score was significantly lower in the cystic group [P =0.002]and correlated with SPAP [r =0.23]. Global HTCT score of 10.3 +/- 2.5 was associated with SPAP >/= 40 mmHg[P =0.011]. Patients with cystic bronchiectasis have significant impairment of pulmonary physiology compared with cylindrical bronchiectasis patients. HRCT score correlated with PFTs and SPAP

Childhood bronchiectasis: a review

More than two decades ago, bronchiectasis unrelated to cystic fibrosis [CF] was termed "Orphan disease", because it had become an uncommon clinical entity among children in the developed world. It is more common among children in lower socioeconomic classes and in developing countries, due to more frequent recurrent respiratory infections, environmental airway irritants, poor immunization, and malnutrition. The pathophysiology includes airway inflammation, mucous production, and regional airway obstruction, but the reasons some children develop bronchiectasis while other do not is not clear. Public measures aimed at improving living conditions for children and prevention of respiratory infections with anti-viral vaccines will have a good impact on childhood bronchiectasis than medical treatment

Bronquiectasias associadas à síndrome de Sjõgren / Bronchiectasis associated to Sjõgren syndrome: case report

Diversas formas de comprometimento pulmonar têm sido descritas na síndrome de Sjõgren, incluindo raros casos em que bronquiectasias foram o achado predominante. Relata-se o caso de uma paciente com história de infecções respiratórias de repetição e dispnéia progressiva de longa evolução, cuja tomografia computadorizada de alta resolução evidenciava inúmeras bronquiectasias. A revisão de uma biópsia pulmonar realizada no início dos sintomas mostrava alterações bronquiolares de natureza inflamatória e fibrótica. Apesar da presença de sintomas de síndrome seca há anos, o diagnóstico de SS foi feito apenas tardiamente. São discutidos os possíveis mecanismos patogenéticos que possam ter levado ao desenvolvimento das bronquiectasias e a necessidade de um apurado senso clínico para o diagnóstico precoce de tais condições.

Bronquiectasias experimentais / Experimental bronchiectasis

Foi efetuado um estudo experimental destinado a avaliar alguns possíveis etiopatogénicos das bronquiectasias. Foram operados 6 grupos de cäes ao nível do hilo do lobo inferior do pulmäo esquerdo, tendo-se efetuado as seguintes intervençöes: 1 - Reimplante do lobo inferior esquerdo; 2 - Despleurizaçäo hilar; 3 - Estenose por compressäo extrínseca do brônquio; 4 - Secçäo da arteria pulmonar; 5 - Estenose da veia pulmonar por compressäo extrínseca; 6 - Associaçäo de estenose do brônquio e da veia pulmonar com secçäo da arteria pulmonar. Os resultados foram avaliados em estudos post-mortem que incluíram exames anátomo-patológicos, angiografias e moldes de injecçäo-corrosäo da árvore brônquica e das estruturas vasculares. Observaram-se bronquiectasias apenas em alguns animais dos grupos 1 e 6, sendo normais as árvores respiratórias de todo os outros. Pode-se concluir que: 1 - o aperto brónquico, atuando isoladamente, näo parece ser fator determinante da sua produçäo; 2 - as dilataçöes brônquicas podem aparecer sem aperto brônquico prévio, sendo de admitir a desnervaçäo como fator condicionante do seu desenvolvimento; 3 - as obstruçöes ou estreitamentos isolados da artéria e da veia pulmonar näo produzem alteraçöes identificáveis na parede brônquica; mas alteraçöes obstrutivas idênticas, atuando sinergicamente nos 3 componentes funcionais do hilo, desencadeiam uma incidência significativa de lesöes broncopulmonares, que incluem as bronquiectasias